Congenital rubella (German measles) syndrome is a well-defined combination of ocular, otologic, and cardiac abnormalities, accompanied by microcephaly and variable developmental delay. The incidence has decreased markedly in North America since widespread vaccination of children was instituted in the late 1960s; however, rubella remains a cause of infant morbidity and mortality in less-developed countries.
Ocular abnormalities include a peculiar nuclear cataract that is sometimes floating in a liquefied lens cortex, glaucoma, microphthalmia, and retinal abnormalities that vary from a subtle salt-and-pepper retinopathy (most common finding; Fig 28-26) to pseudoretinitis pigmentosa. Diagnosis is based on this characteristic clinical picture and is supported by results of serologic testing. The virus itself can be isolated from pharyngeal swabs and from the lens contents at the time of cataract surgery.
Lensectomy is usually required for cataracts. Infected eyes are prone to postoperative inflammation and subsequent secondary membrane formation. Thus, topical steroids and mydriatics should be used aggressively. In adults, rubella virus infection has been identified as a probable cause of Fuchs heterochromic uveitis.
Figure 28-26 Fundus photograph from a 6-year-old patient with rubella syndrome (electroretinography results were normal).
Excerpted from BCSC 2020-2021 series: Section 10 - Glaucoma. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.